We describe the case of an 81 year old woman that presented with jaundice, bile duct dilation, hepatic liver tests abnormalities and elevated levels of CA-19-9 (741 UI). Intestinal and pancreatic neoplasia were ruled out. Hepatic biopsy revealed chronic inflammation, cirrhosis and findings consistent with autoimmune hepatitis. The patient was given a course of corticoids and the disease as well as levels of CA-19-9 returned to normal levels.